Rabbit NEU1 Polyclonal Antibody | anti-NEU1 antibody

NEU1 antibody

Cat. Num. AAA9405904

• Gene Names: NEU1; NEU; NANH; SIAL1
• Reactivity: Human, Mouse, Rat
• Applications: Western Blot
• Purity: Antibodies were purified by affinity purification using immunogen.
• Synonyms: NEU1; Polyclonal Antibody; NEU1 antibody; NEU; NANH; SIAL1; anti-NEU1 antibody

• Host: Rabbit
• Reactivity: Human, Mouse, Rat
• Clonality: Polyclonal
• Specificity: The antibody detects endogenous level of total NEU1 antibody.
• Purity/Purification: Antibodies were purified by affinity purification using immunogen.
• Form/Format: Supplied at 1.0mg/mL in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
• Concentration: 1.0 mg/ml (varies by lot)
• Sequence Length: 415

• Applicable Applications for anti-NEU1 antibody: Western Blot (WB)
• Application Notes: Western blotting: 1:500 - 1:2000
• Immunogen Type: Recombinant Protein
• Immunogen Description: Recombinant protein of human NEU1.
• Target Name: NEU1
• Preparation and Storage: Store at -20 degree C

Western Blot (WB)

(Western blot analysis of extracts of various cell lines, using NEU1 antibody.)

Related Product Information for anti-NEU1 antibody

The protein encoded by this gene is a lysosomal enzyme that cleaves terminal sialic acid residues from substrates such as glycoproteins and glycolipids. In the lysosome, this enzyme is part of a heterotrimeric complex together with beta-galactosidase and cathepsin A (the latter is also referred to as 'protective protein'). Mutations in this gene can lead to sialidosis, a lysosomal storage disease that can be type 1 (cherry red spot-myoclonus syndrome or normosomatic type), which is late-onset, or type 2 (the dysmorphic type), which occurs at an earlier age with increased severity.

Product Categories/Family for anti-NEU1 antibody

Total protein Ab

NCBI and Uniprot Product Information

• NCBI GI #: 4557791
• NCBI GeneID: 4758
• NCBI Accession #: NP_000425.1
• NCBI GenBank Nucleotide #: NM_000434.3
• UniProt Accession #: Q99519
• Molecular Weight: 45,467 Da
• NCBI Official Full Name: sialidase-1
• NCBI Official Synonym Full Names: sialidase 1 (lysosomal sialidase)
• NCBI Official Symbol: NEU1
• NCBI Official Synonym Symbols: NEU; NANH; SIAL1
• NCBI Protein Information: sialidase-1
• UniProt Protein Name: Sialidase-1
• Protein Family: Sialidase
• UniProt Gene Name: NEU1
• UniProt Synonym Gene Names: NANH
• UniProt Entry Name: NEUR1_HUMAN

NCBI Description

The protein encoded by this gene is a lysosomal enzyme that cleaves terminal sialic acid residues from substrates such as glycoproteins and glycolipids. In the lysosome, this enzyme is part of a heterotrimeric complex together with beta-galactosidase and cathepsin A (the latter is also referred to as 'protective protein'). Mutations in this gene can lead to sialidosis, a lysosomal storage disease that can be type 1 (cherry red spot-myoclonus syndrome or normosomatic type), which is late-onset, or type 2 (the dysmorphic type), which occurs at an earlier age with increased severity. [provided by RefSeq, Jul 2008]

Uniprot Description

NEU1: Catalyzes the removal of sialic acid (N-acetylneuramic acid) moities from glycoproteins and glycolipids. To be active, it is strictly dependent on its presence in the multienzyme complex. Appears to have a preference for alpha 2-3 and alpha 2-6 sialyl linkage. Defects in NEU1 are the cause of sialidosis (SIALIDOSIS). It is a lysosomal storage disease occurring as two types with various manifestations. Type 1 sialidosis (cherry red spot-myoclonus syndrome or normosomatic type) is late-onset and it is characterized by the formation of cherry red macular spots in childhood, progressive debilitating myoclonus, insiduous visual loss and rarely ataxia. The diagnosis can be confirmed by the screening of the urine for sialyloligosaccharides. Type 2 sialidosis (also known as dysmorphic type) occurs as several variants of increasing severity with earlier age of onset. It is characterized by the presence of abnormal somatic features including coarse facies and dysostosis multiplex, vertebral deformities, mental retardation, cherry-red spot/myoclonus, sialuria, cytoplasmic vacuolation of peripheral lymphocytes, bone marrow cells and conjunctival epithelial cells. Belongs to the glycosyl hydrolase 33 family.

Protein type: EC 3.2.1.18; Hydrolase; Lipid Metabolism - sphingolipid; Motility/polarity/chemotaxis; Glycan Metabolism - other glycan degradation

Chromosomal Location of Human Ortholog: 6p21.3

Cellular Component: lysosomal lumen; intracellular membrane-bound organelle; lysosome; lysosomal membrane; cytoplasmic membrane-bound vesicle; plasma membrane; cell junction

Molecular Function: exo-alpha-sialidase activity

Biological Process: oligosaccharide catabolic process; sphingolipid metabolic process; cellular protein metabolic process; dolichol-linked oligosaccharide biosynthetic process; protein amino acid N-linked glycosylation via asparagine; glycosphingolipid metabolic process; post-translational protein modification; lipid catabolic process

Disease: Neuraminidase Deficiency